31 Days of Cystic Fibrosis

May is Cystic Fibrosis awareness month, and once again I’ve done 31 Days of Cystic Fibrosis on Facebook and Instagram.
In 2019 I concentrated on a hidden side of CF and profiled people who are impacted by CF.

May 29th – May 31th 2019

31 Days of Cystic Fibrosis. May 29Natalie Cahill: A Wife’s Story

For Natalie Cahill the topic of CF came up on her first date with Michael. “It was the first time we had gone out – we were eating and Michael took his creon. I asked him what they were for and he said, ‘I have Cystic Fibrosis’. I googled it when I got home and it came up with the worst information. As our relationship developed I learnt more about CF from Michael.”

CF affects people in different ways, and Natalie initially thought a relationship with a person with CF would be harder. “Apart from when he is hospitalised, the rest is simply the way things are in our house. Very few times has it stopped us doing anything a non-CF house does.”

The decision to start a family, however, was one of those times. Although men with CF can father biological children, for most men with CF, fertility assistance is required. “We had to conceive through IVF. Our miracle daughter Zoe was conceived on our 11th cycle.”

Natalie is very pragmatic about the challenges of life with CF. “When Michael is hospitalised, I have to balance seeing him and getting Zoe to school for her after school activities to keep her life ‘normal’. We do have family that can help out where possible. Nothing in life is guaranteed, so I go with it and what life throws at us as a family. We are lucky Michael is pretty ‘healthy’. Which has allowed us to travel a little.”

“Michael is an incredible person – he isn’t CF, but it is a part of him. He has a strength that amazes me every day. Along with the ability to get on with life no matter what CF throws at him. Michael always looks after Zoe and I while balancing work and going to the gym to keep healthy and fit.”

31 Days of Cystic Fibrosis. May 30Tanya Farnell Brown: Dual Perspective, From Sister to Mother

CF has always been a part of Tanya Farnell Brown’s life. Her two younger sisters, Lee-Anne and Narelle, were diagnosed with CF shortly after they were born. “Growing up, it seemed like we were always at the hospital. Chest infections and coughing was the norm in our house. I was often sick, but I noticed both the girls doing things I didn’t. They had this smelly powdery stuff called Vicase that they’d mix with Apple sauce at mealtimes, and physio and nebs too. Lappa or Judy would come to the house to help mum with their physio. They’d take turns. Do physio then nebs. No one ever wanted to be first.”

When Tanya was 13, she started to notice the difference in Lee-Anne’s health. “Narelle was always the healthier of the two and never had any hospital stays, whereas Lee-Anne’s health was up and down; she was in and out of hospital. Hospital stays started to became more frequent.”

“We lost Lee-Anne at the age of 18. She had a low lung function for a long time, along with other issues. This time her kidneys started to fail. Treatment wasn’t successful, and there was talk of her going over east for transplant, as it couldn’t be done here in WA at that time. Sadly it was too late, and she became too ill to be able to travel there for it.”

“Narelle is 43 now and Mum to a 13 year-old son. She is one of the strongest and bravest women I know. She had the opportunity to be a part of the Orkambi trials, and it is now part of her treatment plan. I’m happy to know it seems to be working for her. She is stable at the moment and hasn’t had to have IV treatments for over a year!”

“We were devastated to lose my sister Lee-Anne. And I definitely don’t want to lose another sister.”

In 1998 Tanya gave birth to her son, Sam. “Sam was a happy, healthy baby showing no health concerns. For some reason, CF was in the back of my mind. I looked out for signs. Over the summer months, I noticed a saltiness to his skin. Not just his forehead but his feet. I thought maybe I was overthinking it. Was I making something out of nothing? It could be nothing, but it still stayed in my head. Sam was a good eater gobbled up everything. But I felt I wanted to go to the hospital and have him checked, for my piece of mind.”

The diagnosis was soon confirmed. Like Tanya’s younger sisters, Sam too had CF. “I was given info packs to read and told ‘Now go home and enjoy him for as long as you have him. Someone will be in touch for further testing.’ This, of course, was emotionally overwhelming. I’d lost my sister, Lee-Anne, just 6 years before from CF.”

The daily routine of medications, nebs and physio started again, but this time, it was Tanya’s responsibility. “My main aim was prevention – to prevent his exposure to illness. I became super vigilant. I was always listening to his breathing and looking for signs that might show he was coming down with something. I started screening visitors. I’d ask them all if they were sick before they came over. Or before we went to theirs. I quickly learnt that other people’s perceptions of sick are definitely different from mine. They don’t realise how the risk of a simple cold to them could end up being life threatening to another.”

“I was strict with a physio routine. As a baby laying on our laps, it was nice when Sam stayed still. We had Judy as a physio aid for us from CFWA. Judy had also done Lee-Anne and Narelle’s physio when they were younger. To me, it was like an old family member coming home.”

For Sam, hospital admissions were stressful, and doing IV treatments at home became the preferred treatment option. “Sam is now 20 and he’s not needed IV treatment for 12 years. We’ve been lucky to be able to keep his cough dry, and he’s only chesty when he has an infection.”

“I’ve learnt to take each day as it comes. Be thankful for every day that we are, in our own way, healthy and happy. Each day try to look on the good side of things and be hopeful all goes well. All the changes I’ve seen in treatments for CF and the knowledge gained through research over the years is really quite amazing. Like Orkambi. What a gift that is. It reinforces our hopes for the future.”

31 Days of Cystic Fibrosis. May 31
Harry Sippel: A Son’s Story

The implications of what CF meant for Harry Sippel’s mother became apparent at a very young age as he would accompany her to her various doctor and hospital clinic appointments. “My Mum is a very honest person and would tell me everything that went on with her health. That’s how you must be with such an erratic disease. Even though I was very young, I understood what my Mum had to do in day to day life.”

Rather than struggling with this information, Harry found his Mum’s honesty empowering. “If something bad happened I wouldn’t know what to do or what was going on. If I didn’t know what was going on, why she did certain things, then I’d be angry.”

“I played heaps of sport throughout my life, and thanks to my loving parents they always got me there. Sometimes I was a couple of minutes late for the game because I had to wait for my mum to finish her drugs. Most days, Mum would be there for me; however, if she couldn’t come, Dad would take me. That’s okay because I understood.”

Having watched the ups and downs of CF, Harry’s experienced many emotions. “Over the years, I have been very scared and worried. However, my Mum is the toughest person I know. She taught me how to be positive when times are tough, which is easier for me to deal with it.”

That positivity saw Kerry recently celebrate her 50th birthday.

Harry said, dealing with his mother’s CF’s is the reason he is tough and honest himself. “Everything in life is valuable, and every memory counts. Without my Mum, god knows where I would be today. She taught me how to deal with my type 1 diabetes. Life may throw many hurdles at you, but it’s how you jump those hurdles that really counts.”

31 Days of Cystic Fibrosis. Thankyous!

This year through May I wanted to show that CF is a disease that affects not only the person with CF, but their entire support network. I wouldn’t have been able to do this without the help of the following people:

May is Cystic Fibrosis awareness month, and once again I’ve done 31 Days of Cystic Fibrosis on Facebook and Instagram.
In 2019 I concentrated on a hidden side of CF and profiled people who are impacted by CF.

May 24th – May 28th 2019

31 Days of Cystic Fibrosis. May 24Sixty Five Roses Day

Some dates and celebrations have a special meaning – in May 2011, for me, Sixty Five Roses Day became something more than a day to raise awareness and funds for CF.
Friday, May 27th 2011, started as just another day for me. I knew it was Sixty-Five Roses Day, but I was housebound, dependant on oxygen, and not in any shape to help paint the town red for CF. I was barely able to breathe, despite the oxygen whirling up my nose.

That day changed my life. At 9.30am, I received a call telling me to come to the hospital ASAP, as donated lungs were waiting for me. By 12.20pm, I was prepped and waiting for the last inspection of the donated lungs to take place.

As I lay on the operating table, I thought of many, many things. I ticked off the things on my bucket list I had already accomplished. And I started a new bucket list, for the things I would do. One of the things that sat in both columns was my book, ‘The Mystery of the Sixty-Five Roses’. I’d started writing it back in 2007, and had delivered the completed manuscript to CFWA at the end of 2010. I had indeed written my book. I knew that if I died, my book would continue to go to print.

But I still wanted to hold a printed copy in my hands.

All up, it took six years and a new set of lungs but on February 26th 2013 ‘The Mystery of the Sixty-Five Roses’ was officially launched.

Claire Stubber met Ian during her first session of pre-transplant gym at the Alfred Hospital. “Like most CFs I know Ian didn’t let on that there was anything wrong with him besides a pretty nasty cough that he couldn’t hide and obviously damaged lungs. I remember his pancreatic enzymes because one of my sisters used to pull the capsule apart anddisperse the spheres as we were sitting and talking.”

“After meeting Ian, one of my sisters decided to do her Year 12 science project on Cystic Fibrosis, and so I learnt a bit more about the genetic mutations then and the physical effects that weren’t that obvious to me at the time.”

Four months after the two met, Ian received his call for transplant. “I was in shock, worried but extremely happy for him. I walked around in a fog for the entire day. When I got to the hospital the next day, one of the staff who knew us both well grabbed my hand and pulled me down two flights of stairs so I could see him in ICU. Ian was sitting up, he looked a bit swollen but very pink, and he had a huge grin on his face. Holding his warm hand was amazing. He only spent 24 hours in ICU; he boasts that was a record.”

“I visited him every day on the ward and saw how quickly he progressed, and despite a faulty epidural and other problems, he was cheerful. I think seeing the process of transplantation first hand was enormously reassuring; I was there the day he had his first and only bout of rejection, he was practically sitting on top of the heater because he was shivering from fever. There were procedures and setbacks that I witnessed that helped me to quickly put things in perspective when they later happened to me.”

Claire received her call for transplant seven months later. At the time, Ian had returned to Perth. “I came around four or five days after my transplant in the ICU, so I woke up to the news that Ian was on his way over. He spent two weeks with me, and it was lovely, I learnt then that he would love me through anything.”

On returning to Perth, Claire experienced health problems. Twelve months went by, and the cause remained mysterious. “My father (a consultant physician) suggested I go to Melbourne for help. We stayed for three months initially, but they identified the problem within days. It seemed a simple decision (apart from leaving loved ones) after that to make the move.”

“I was worried about moving to Melbourne and away from family support when I was hospitalised, but I needn’t have been, I was sick or close to dying for most of last year, and Ian just seemed to know exactly what to do and what I needed when I needed it. Ian introduced me to a world in which there could be wordless communication about how one was feeling and what one had experienced, not just between us but to others, mainly people with CF, who had similar experiences. I seemed to be always accepted as not having CF but still part of the CF community. Luckily there are few times when Ian and I are ill at the same time. I think it makes things easier; we don’t have to explain a lot of things to each other.”

“The physical effects of CF can be horrendous, and transplant does not solve them all, in Ian’s case, it has made most of them more bearable. Ian claims CF as a large part of his identity in a positive way and often points out that his various character traits do have a connection to the fact that he has CF. He showed me so many times how to deal with illness with humour. He very rarely complains. He is generous in many ways and has overcome so much that I am in awe of him.”

The generosity of two courageous families who decided to donate their loved one organs changed both their lives. Combined, the extra time Claire and Ian have been gifted equals 46 years.

Ian is a renowned Cosplay photographer, you can follow him on Instagram

31 Days of Cystic Fibrosis. May 27
Aidan Fraser: A Husband’s Story

Sharon Lawrence I Clinical Nurse Consultant

Statistically, CF patients as a group, have a longer median survival post-transplant compared to other lung transplant recipients. Sharon Lawrence explains the many factors that contribute to this.
“Many people with respiratory problems are referred later in life, and come to end-stage respiratory disease with comorbidity factors. CF patients can be referred any time between the ages of 10 – 50. Although CF patients are living longer and better, and their age to transplant is becoming later, they are still the youngest population of patients that come to transplant.”

“CF patients have life-long managed their illness. When they get to transplant, they understand the reality of changing treatment options from one illness to the treatment options of another. They don’t come to transplant with the expectation of miraculous results.”

“Most CF patients take to complex medications regimes quite well, and although there are somewhere between 10 – 15 medications they need to take, it’s not as aggressive as the medication plan they were on pre-transplant with the nebs and all that chest physio. Treatment is not as onerous- and they get to live their life between morning and evening medications.”

“CF Patients take opportunities they didn’t have before, now that they don’t have the burden of the lung disease component of CF – yes they still have CF, but not the treatment burden that CF lungs require. I enjoy transplant for CF patients because of the benefits and because of what it means to them and also for their families.”

31 Days of Cystic Fibrosis. May 28
Steph Anderson: A Friend’s Story

Steph Anderson and Neely began their friendship in high school. “Neely was always open about her CF. She was coughing one day, quite severely. I asked why? She said quickly. I’ve got CF, and I’ll be dead by the time I’m 27. So I went home, and I googled it.”
As their friendship developed, the pair started hanging out outside of school. “We were at Neely’s house one day after school and Lappa, the physio, showed up and set up the table.” Steph still recalls the shock she felt. “The next thing he’s beating the crap out of her back.”Steph had health problems also, and on occasion, the pair would even be in PMH at the same time. However, it was when the girls were in their twenties that Steph first noticed the decline in Neely’s health. “Neely started saying she couldn’t go out because was feeling unwell or had landed in hospital. It wasn’t until the subject of organ donation came up that it really hit home.”Five years after Neely’s lung transplant, Steph underwent a kidney transplant. “Neely was a great help explaining the different medications and what side effects came with them.”While Steph’s new kidney was functioning well, Neely was undergoing a tough battle. After surviving bowel cancer and it’s various complications, the medications, Neely required to prevent her lungs from rejecting caused damage to her kidneys.The last year of Neely’s life was an emotional journey. “Me having the healthy kidney, and her needing one, made me feel survivor’s guilt, I wish it was like a liver so I could have shared it with her.”

“I’ve had friends die before, but nothing impacted me like watching Neely. I could do practical things, be there and listen to her. After TAFE I’d go straight to the hospital and leave at 2 am. She fought hard, but she was also my age. I knew she wasn’t going to have a long life, but she should have. She should have had the chance to have children and grow old.”

Steph continues to fundraise for CF, recently participating in the HBF Run for a Reason and selling roses on Sixty Five Roses Day. “I fundraise as a way of working through my grief.”

“The world is a different place without her. Sadder. She’s left a massive, massive hole.”

May is Cystic Fibrosis awareness month, and once again I’ve done 31 Days of Cystic Fibrosis on Facebook and Instagram.
In 2019 I concentrated on a hidden side of CF and profiled people who are impacted by CF.

May 19th – May 23rd 2019

31 Days of Cystic Fibrosis. May 19 – 21Barbara Jordan: A Mother’s Story

Barbara Jordan worried her way through her second pregnancy. Her first child, Melinda, had been diagnosed with CF when she was two months old. Melinda’s diagnosis came hot on the heels of the news that a relative in the UK had lost her son to CF.
Diagnosed with CF at birth, Julie’s survival was initially predicted to be less than 48 hours. Despite the dire prediction, Julie did survive, and soon, Barbara’s daily routine saw her deal with the medical requirements of two children with CF.

Barbara knew life would be busy with double the number of treatments and hospital visits. “I thought they would help each other understand what they needed to do, and I do feel that having the two girls close together was helpful. They would talk to each other during treatments. The girls had a great connection because of their shared experiences.”

Julie always struggled more than Melinda and subsequently spent more time in hospital. “Melinda was maybe about eight when she told me that I loved Julie more. I needed to explain that Julie was sicker, and consequently needed more attention.”

As a young adult, Julie’s health continued to decline, and eventually, she told Barbara that the team at Sir Charlies Gairdner Hospital, headed by Dr Gerrad Ryan, had recommended Julie as a candidate for a transplant.

With Julie on the path to transplant, life became more daunting for Barbara. The move to Melbourne, while Julie was waitlisted for transplant, meant Barbara needed to leave Melinda behind. “Melinda was living her own life, and I knew that this was the best thing for Julie.”

Initially, on their arrival in Melbourne, Barbara and Julie were across the road from the hospital. Later they shifted into a CF house. Barbara describes the night Julie received the call to come to the hospital for transplant as a moment that was both frightening and filled with happiness.
Extensive roadworks caused one of the main roads to be closed off. Once Barbara explained the situation and the urgency of getting to the hospital, the workmen let her though, and she continued on to the hospital driving on the wrong side of the road.

Barbara felt as if a weight had lifted from her shoulders when she received the call informing her that Julie was out of surgery. “I was over the moon,” Barbara said.

Julie’s recovery went well; Barbara describes her recovery as “Unbelievable. She improved so much. She was breathing and laughing and not having difficulty with any of it.”

Until a rigid bronch, a procedure that visualizes the trachea and proximal bronchi, was performed. “I received a terrifying phone call in the middle of the night. The doctors put Julie under too far. They had to use the paddles to bring her back to life.”

Despite the setback, Julie was soon ready to return to Perth. Once they were home, Barbara discovered Julie didn’t ‘fit’ into the system anywhere. “Julie was no longer being cared for by the CF team, and it was hard to get the new team to listen.”

Then Julie’s new lungs showed signs of rejection.

“We were in clinic, and the transplant team told Julie there was nothing they could do for her. Julie was asked if she wanted a second transplant. She said, “No. No. I don’t want to put myself, my family, and my friends through what I’ve just been through again. With no guarantee that it is going to be successful. So no. Take me off all my medications and let me go peacefully.”

“They were her words, her choices; she was an adult, it was her body; it was her decision.”

“Melinda moved to NSW – it was hard because it was like I lost both girls at the same time, but I knew that was what Melinda needed to do. She had a dream to have a family, and it wasn’t my place to stop her.”
Barbara Jordan describes the news that Melinda was pregnant as initially devastating, but also exciting. Her main worry was how the pregnancy could impact Melinda. “Back when the girls were diagnosed with CF, I never would have dreamed that I could become a grandmother. Melinda went through so much to have Luca.”For Barbara, Melinda’s transplant journey was different to the hands-on experience of Julie’s transplant. With Melinda living interstate and married, Barbara now relied on the information that was passed on. “I hadn’t realised quite how bad Melinda’s decline had been. I saw Melinda right before she went in for the first transplant. Seeing her sit up with colour in her face afterwards was just unbelievable.”After a rollercoaster ride, with multiple hiccups, Melinda required a second transplant two years ago. “Melinda’s a stubborn girl, and I know she’ll fight her way through whatever CF throws her way for as long as she can. She wants to see Luca marry and have babies all of her own. I know Melinda will be alright.”“Luca has so much of Julie in her it’s incredible to look at. And that is beautiful.”

31 Days of Cystic Fibrosis. May 22Karen De Lore – Business Development Manager, Cystic Fibrosis WA

Karen De Lore came to CFWA with some knowledge of CF, and how the disease was managed. “I felt an empathy for the CF community before I arrived, I thought working for CFWA would provide a cause I could be passionate about. And it is easy to be passionate about CF. Although CF is chronic and awful, the CF community is great.”

As Business Development Manager, Karen is responsible for both fundraising and marketing awareness of CF. “Fundraising, by its nature, also brings a lot of awareness. Both the 65 Roses campaign and Orkambi campaign were targeted towards raising awareness of the battles that families affected by CF face every day. These campaigns bring increased awareness towards understanding the challenges people with CF face in their life.” Karen said.

For people with CF, there is a huge benefit to offering services as an adjunct to hospital services. These focused services not only help relieve the burden on the hospital system; they directly improve the lives of people with CF and their families. CFWA provides additional services on top of those paid for via the Health Department contract – that’s where Karen’s role is vital. Without fundraising, CFWA can’t deliver focussed services in an effective manner.

“Working in the not-for-profit sector in this climate is challenging – asking for money from any part of corporate WA or from the community is sometimes hard – it makes it a battle to secure new funding. Sometimes people want to give money for specific purposes. However, both caring for people with CF and providing funds for research goes hand in hand. It’s no good channelling all the money to research while people are battling and need service delivery.”

31 Days of Cystic Fibrosis. May 23
Aidan Fraser: A Husband’s Story

Meeting Jackie was Aidan Fraser’s first introduction to CF. “Jackie was coping with CF quite well, and it wasn’t evident at all that she was sick. So I was probably a bit naive toward the whole thing . I never imagined it would be this hard, difficult or sad – mostly due to my ignorance as to what CF really was.”
The journey for Aidan has been a learning curve between new routines and methods that can help improve Jackie’s quality of life. “It’s something we deal with and adapt to. This is the new ‘normal’ for us, and you do what you have to do.”
The reality of Jackie’s medical team recommending her for transplant knocked Aidan for six. “It was like being hit with a bat! To hear the words is something you can’t prepare for. Especially when you felt it was still some time away.”

A double lung transplant is not a cure for CF; it is a procedure that trades one set of health problems for another. In an effort to improve Jackie’s quality of life, the couple decided to postpone being waitlisted for transplant while they put in a concentrated effort to focus on Jackie’s health.

Right now, they’re in the midst of a trip around Australia with their dog. It’s a journey they’re taking day by day, constantly reassessing Jackie’s health.

“I can’t explain enough how dealing with CF has changed and enriched my life. Jackie has made me see the world in a different way than I did before knowing her. The biggest thing being compassion and kindness. Without a doubt, she is the kindest, most compassionate person I have ever met. Jackie’s zest for life is contagious, and she has passed all this on to me.”